{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T01:11:25Z","timestamp":1789780285180,"version":"4.0.1"},"reference-count":50,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2024,7,11]],"date-time":"2024-07-11T00:00:00Z","timestamp":1720656000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["NS045630"],"award-info":[{"award-number":["NS045630"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["NS110627"],"award-info":[{"award-number":["NS110627"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["NS125928"],"award-info":[{"award-number":["NS125928"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["NS134493"],"award-info":[{"award-number":["NS134493"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Glia"],"published-print":{"date-parts":[[2024,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Rapid nerve conduction in the peripheral nervous system (PNS) is facilitated by the multilamellar myelin sheath encasing many axons of peripheral nerves. Charcot\u2010Marie\u2010Tooth type 1A (CMT1A), and hereditary neuropathy with liability to pressure palsy (HNPP) are common demyelinating inherited peripheral neuropathies and are caused by mutations in the peripheral myelin protein 22 (PMP22) gene. Duplication of <jats:italic>PMP22<\/jats:italic> leads to its overexpression and causes CMT1A, while its deletion results in <jats:italic>PMP22<\/jats:italic> under expression and causes HNPP. Here, we investigated novel targets for modulating the protein level of PMP22 in HNPP. We found that genetic attenuation of the transcriptional coactivator <jats:italic>Yap<\/jats:italic> in Schwann cells reduces p\u2010TAZ levels, increased TAZ activity, and increases PMP22 in peripheral nerves. Based on these findings, we ablated <jats:italic>Yap<\/jats:italic> alleles in Schwann cells of the <jats:italic>Pmp22<\/jats:italic>\u2010haploinsufficient mouse model of HNPP and identified fewer tomacula on morphological assessment and improved nerve conduction in peripheral nerves. These findings suggest YAP modulation may be a new avenue for treatment of HNPP.<\/jats:p>","DOI":"10.1002\/glia.24592","type":"journal-article","created":{"date-parts":[[2024,7,11]],"date-time":"2024-07-11T10:01:28Z","timestamp":1720692088000},"page":"1974-1984","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Loss of <scp>YAP<\/scp> in Schwann cells improves <scp>HNPP<\/scp> pathophysiology"],"prefix":"10.1002","volume":"72","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9488-3905","authenticated-orcid":false,"given":"Seth M.","family":"Moore","sequence":"first","affiliation":[{"name":"Department of Biochemistry University at Buffalo  Buffalo New York USA"},{"name":"Institute for Myelin and Glia Exploration, Jacob's School of Medicine and Biomedical Sciences University at Buffalo  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eunbi","family":"Jeong","sequence":"additional","affiliation":[{"name":"Department of Biochemistry University at Buffalo  Buffalo New York USA"},{"name":"Institute for Myelin and Glia Exploration, Jacob's School of Medicine and Biomedical Sciences University at Buffalo  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muhammad","family":"Zahid","sequence":"additional","affiliation":[{"name":"Institute for Myelin and Glia Exploration, Jacob's School of Medicine and Biomedical Sciences University at Buffalo  Buffalo New York USA"},{"name":"Department of Biological Sciences University at Buffalo  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Gawron","sequence":"additional","affiliation":[{"name":"Department of Biochemistry University at Buffalo  Buffalo New York USA"},{"name":"Institute for Myelin and Glia Exploration, Jacob's School of Medicine and Biomedical Sciences University at Buffalo  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simar","family":"Arora","sequence":"additional","affiliation":[{"name":"Albany Medical College Department of Neuroscience and Experimental Therapeutics  Albany New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Belin","sequence":"additional","affiliation":[{"name":"Albany Medical College Department of Neuroscience and Experimental Therapeutics  Albany New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3942-6637","authenticated-orcid":false,"given":"Fraser","family":"Sim","sequence":"additional","affiliation":[{"name":"Department of Pharmacology and Toxicology University at Buffalo  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9868-1569","authenticated-orcid":false,"given":"Yannick","family":"Poitelon","sequence":"additional","affiliation":[{"name":"Albany Medical College Department of Neuroscience and Experimental Therapeutics  Albany New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2276-9182","authenticated-orcid":false,"given":"M. Laura","family":"Feltri","sequence":"additional","affiliation":[{"name":"Department of Biochemistry University at Buffalo  Buffalo New York USA"},{"name":"Institute for Myelin and Glia Exploration, Jacob's School of Medicine and Biomedical Sciences University at Buffalo  Buffalo New York USA"},{"name":"Department of Neurology University at Buffalo  Buffalo New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,7,11]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.17-12-04662.1997"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1195-274"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.21118"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.4264-09.2010"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.15252\/emmm.201606349"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/nchembio.2036"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(93)90058-X"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13238-010-0138-3"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.conb.2006.08.005"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms15161"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/0378-1119(93)90384-F"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/jns.12431"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1074\/JBC.M115.692285"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1999.tb08595.x"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.20982"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.24086"},{"issue":"7","key":"e_1_2_8_18_1","first-page":"2085","article-title":"Comparative gene expression profile analysis of neurofibromatosis 1\u2010associated and sporadic pilocytic astrocytomas","volume":"62","author":"Gutmann D. H.","year":"2002","journal-title":"Cancer Research"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/nm.4483"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/glia.24508"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1006290"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccr.2014.01.010"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.5893-10.2011"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-017-02219-9"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/19.24.6778"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.23775"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1038\/s44321-023-00019-5"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.1908-14.2014"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajpath.2017.10.021"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.4103\/1673-5374.141800"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddw158"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1002\/glia.22855"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1002\/glia.22852"},{"key":"e_1_2_8_34_1","unstructured":"Morell P. &Quarles R. H.(1999).Characteristic Composition of Myelin. Retrieved fromhttps:\/\/www.ncbi.nlm.nih.gov\/books\/NBK28221\/"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1038\/s42255-022-00584-y"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddy191"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0692-159"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.4161\/CBT.23788"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1038\/nn.4316"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1111\/jnc.14295"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02736858"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1038\/srep07286"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1093\/BRAIN\/124.11.2177"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1093\/BRAIN\/AWV241"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-7216-9491-7.50072-7"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semcdb.2012.05.002"},{"issue":"8","key":"e_1_2_8_47_1","first-page":"2145","article-title":"Yes\u2010associated protein (YAP65) is a proline\u2010rich phosphoprotein that binds to the SH3 domain of the yes proto\u2010oncogene product","volume":"9","author":"Sudol M.","year":"1994","journal-title":"Oncogene"},{"key":"e_1_2_8_48_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-9-38"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.1101\/gad.888601"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.1002\/dneu.22744"},{"key":"e_1_2_8_51_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccell.2018.01.005"}],"container-title":["Glia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/glia.24592","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,11,20]],"date-time":"2024-11-20T09:54:54Z","timestamp":1732096494000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/glia.24592"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,7,11]]},"references-count":50,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2024,11]]}},"alternative-id":["10.1002\/glia.24592"],"URL":"https:\/\/doi.org\/10.1002\/glia.24592","archive":["Portico"],"relation":{},"ISSN":["0894-1491","1098-1136"],"issn-type":[{"value":"0894-1491","type":"print"},{"value":"1098-1136","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,7,11]]},"assertion":[{"value":"2024-02-29","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-06-29","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-07-11","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}